Mitochondrial functional alterations in relation to pathophysiology of Huntington's disease

scientific article published on June 2010

Mitochondrial functional alterations in relation to pathophysiology of Huntington's disease is …
instance of (P31):
review articleQ7318358
scholarly articleQ13442814

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P356DOI10.1007/S10863-010-9288-5
P698PubMed publication ID20464463

P2093author name stringKochupurackal P Mohanakumar
Mritunjay Pandey
Rajamma Usha
P2860cites workPeroxisome proliferator-activated receptor-gamma coactivator 1 alpha (PGC-1 alpha): transcriptional coactivator and metabolic regulatorQ24294798
Huntingtin promotes cell survival by preventing Pak2 cleavageQ24314724
Towards a transgenic model of Huntington's disease in a non-human primateQ24644013
A novel gene containing a trinucleotide repeat that is expanded and unstable on Huntington's disease chromosomes.Q27860836
Inhibition of calpain cleavage of huntingtin reduces toxicity: accumulation of calpain/caspase fragments in the nucleusQ28505053
Mitochondrial cyclic AMP response element-binding protein (CREB) mediates mitochondrial gene expression and neuronal survivalQ28580488
Mutant huntingtin impairs axonal trafficking in mammalian neurons in vivo and in vitroQ28584697
Changes in cortical and striatal neurons predict behavioral and electrophysiological abnormalities in a transgenic murine model of Huntington's diseaseQ28589793
A Huntington's disease CAG expansion at the murine Hdh locus is unstable and associated with behavioural abnormalities in miceQ28594336
Exon 1 of the HD gene with an expanded CAG repeat is sufficient to cause a progressive neurological phenotype in transgenic miceQ29615357
Early degenerative changes in transgenic mice expressing mutant huntingtin involve dendritic abnormalities but no impairment of mitochondrial energy productionQ45302993
Mitochondrial dysfunction in Huntington's disease: the bioenergetics of isolated and in situ mitochondria from transgenic miceQ45304684
Clinical correlates of mitochondrial function in Huntington's disease muscleQ45305337
Cytochrome C and caspase-9 expression in Huntington's diseaseQ45306362
Mitochondrial NAD+-linked State 3 respiration and complex-I activity are compromised in the cerebral cortex of 3-nitropropionic acid-induced rat model of Huntington's disease.Q45306770
Oxidative stress parameters in plasma of Huntington's disease patients, asymptomatic Huntington's disease gene carriers and healthy subjects : a cross-sectional studyQ45306906
Mitochondrial sensitivity and altered calcium handling underlie enhanced NMDA-induced apoptosis in YAC128 model of Huntington's disease.Q45307199
DNA End Labeling (TUNEL) in Huntington's Disease and Other Neuropathological ConditionsQ45307545
Caspase-dependent and -independent cell death induced by 3-nitropropionic acid in rat cortical neurons.Q46859922
Expression of the Huntington's disease gene is regulated in astrocytes in the arcuate nucleus of the hypothalamus of postpartum ratsQ48204539
Functional anatomy of the basal ganglia. I. The cortico-basal ganglia-thalamo-cortical loopQ56093340
Neurochemical and histologic characterization of striatal excitotoxic lesions produced by the mitochondrial toxin 3-nitropropionic acidQ72098398
Mitochondrial aconitase is a source of hydroxyl radical. An electron spin resonance investigationQ73754845
Mitochondrial swelling impairs the transport of organelles in cerebellar granule neuronsQ81038733
Increased apoptosis of Huntington disease lymphoblasts associated with repeat length-dependent mitochondrial depolarizationQ95810356
Huntingtin bodies sequester vesicle-associated proteins by a polyproline-dependent interaction.Q30164317
Characterization of cybrid cell lines containing mtDNA from Huntington's disease patients.Q30304195
Relationships among molecular genetic and respiratory properties of Parkinson's disease cybrid cells show similarities to Parkinson's brain tissuesQ30437451
Reduced axonal transport in Parkinson's disease cybrid neurites is restored by light therapyQ30439165
Chronic, low-dose rotenone reproduces Lewy neurites found in early stages of Parkinson's disease, reduces mitochondrial movement and slowly kills differentiated SH-SY5Y neural cellsQ30440337
N-terminal mutant huntingtin associates with mitochondria and impairs mitochondrial traffickingQ30486372
Effects of overexpression of huntingtin proteins on mitochondrial integrityQ30489322
Inhibitors of cytochrome c release with therapeutic potential for Huntington's diseaseQ30847955
Apoptotic mode of cell death in substantia nigra following intranigral infusion of the parkinsonian neurotoxin, MPP+ in Sprague-Dawley rats: cellular, molecular and ultrastructural evidencesQ33280786
Unraveling a role for dopamine in Huntington's disease: the dual role of reactive oxygen species and D2 receptor stimulationQ33922831
Loss of mitochondrial membrane potential is associated with increase in mitochondrial volume: physiological role in neurones.Q33989454
Chronic mitochondrial energy impairment produces selective striatal degeneration and abnormal choreiform movements in primatesQ34103541
Glutamate transporters in neurologic diseaseQ34187611
Replication of the neurochemical characteristics of Huntington's disease by quinolinic acidQ34384772
Involvement of mitochondrial complex II defects in neuronal death produced by N-terminus fragment of mutated huntingtinQ34483577
Ca(2+) signalling in mitochondria: mechanism and role in physiology and pathology.Q35196252
Reactive oxygen species and the central nervous systemQ35389986
Selective defect of in vivo glycolysis in early Huntington's disease striatumQ35634060
Mitochondrial DNA damage is a hallmark of chemically induced and the R6/2 transgenic model of Huntington's diseaseQ35706627
Phenotypic characterization of individuals with 30-40 CAG repeats in the Huntington disease (HD) gene reveals HD cases with 36 repeats and apparently normal elderly individuals with 36-39 repeatsQ35882893
Sustained elevation of extracellular dopamine causes motor dysfunction and selective degeneration of striatal GABAergic neurons.Q35978877
Expression of mutant huntingtin in glial cells contributes to neuronal excitotoxicityQ36320596
Regulation of mitochondrial matrix volumeQ36548160
Aggregation of huntingtin in yeast varies with the length of the polyglutamine expansion and the expression of chaperone proteinsQ37109961
Impaired PGC-1alpha function in muscle in Huntington's disease.Q37323316
Mitochondrial structural and functional dynamics in Huntington's diseaseQ37356257
Impaired mitochondrial trafficking in Huntington's diseaseQ37466100
Duplication of biochemical changes of Huntington's chorea by intrastriatal injections of glutamic and kainic acidsQ39114293
Mitochondrial-dependent apoptosis in Huntington's disease human cybrids.Q39751441
Calcium homeostasis and mitochondrial dysfunction in striatal neurons of Huntington diseaseQ40031554
Ubiquitin-proteasome system alterations in a striatal cell model of Huntington's diseaseQ40141779
Extended polyglutamine repeats trigger a feedback loop involving the mitochondrial complex III, the proteasome and huntingtin aggregatesQ40159836
Increased oxidative damage and mitochondrial abnormalities in the peripheral blood of Huntington's disease patientsQ40207281
Mutant huntingtin expression induces mitochondrial calcium handling defects in clonal striatal cells: functional consequencesQ40231756
Oxidative stress promotes mutant huntingtin aggregation and mutant huntingtin-dependent cell death by mimicking proteasomal malfunction.Q40317881
Severe ultrastructural mitochondrial changes in lymphoblasts homozygous for Huntington disease mutationQ40351019
Mutant huntingtin directly increases susceptibility of mitochondria to the calcium-induced permeability transition and cytochrome c releaseQ40551652
Huntington's disease, energy, and excitotoxicityQ40580788
Early mitochondrial calcium defects in Huntington's disease are a direct effect of polyglutamines.Q40721588
Calpain activation in Huntington's disease.Q40723358
Altered proteasomal function due to the expression of polyglutamine-expanded truncated N-terminal huntingtin induces apoptosis by caspase activation through mitochondrial cytochrome c releaseQ40808988
Huntingtin's neuroprotective activity occurs via inhibition of procaspase-9 processingQ40817529
A cellular model that recapitulates major pathogenic steps of Huntington's diseaseQ41017315
Regional mitochondrial respiratory activity in Huntington's disease brainQ41360902
Enhanced sensitivity to N-methyl-D-aspartate receptor activation in transgenic and knockin mouse models of Huntington's diseaseQ41698021
Complex II inhibition by 3-NP causes mitochondrial fragmentation and neuronal cell death via an NMDA- and ROS-dependent pathway.Q41969689
Oxidative damage and metabolic dysfunction in Huntington's disease: selective vulnerability of the basal ganglia.Q42438521
Widespread expression of the human and rat Huntington's disease gene in brain and nonneural tissuesQ42503942
Metabolic changes in the basal ganglia of patients with Huntington's disease: an in situ hybridization study of cytochrome oxidase subunit I mRNA.Q42518943
Dopamine determines the vulnerability of striatal neurons to the N-terminal fragment of mutant huntingtin through the regulation of mitochondrial complex II.Q43206399
L-DOPA-induced 6-hydroxydopamine production in the striata of rodents is sensitive to the degree of denervationQ43237341
Increased oxidative damage to DNA in a transgenic mouse model of Huntington's diseaseQ43830045
Specific progressive cAMP reduction implicates energy deficit in presymptomatic Huntington's disease knock-in miceQ44316963
The first 17 amino acids of Huntingtin modulate its sub-cellular localization, aggregation and effects on calcium homeostasis.Q44376761
Calpain is a major cell death effector in selective striatal degeneration induced in vivo by 3-nitropropionate: implications for Huntington's disease.Q44495460
In vivo calpain/caspase cross-talk during 3-nitropropionic acid-induced striatal degeneration: implication of a calpain-mediated cleavage of active caspase-3.Q44548881
Disruption of Axonal Transport by Loss of Huntingtin or Expression of Pathogenic PolyQ Proteins in DrosophilaQ44607268
Proteomic and oxidative stress analysis in human brain samples of Huntington disease.Q44737699
Mitochondrial calcium, oxidative stress and apoptosis in a neurodegenerative disease model induced by 3-nitropropionic acidQ44792354
Mitochondrial defect in Huntington's disease caudate nucleusQ45291734
In situ mitochondrial Ca2+ buffering differences of intact neurons and astrocytes from cortex and striatum.Q45292096
Cytochrome c oxidase isoform IV-2 is involved in 3-nitropropionic acid-induced toxicity in striatal astrocytesQ45293168
Striatal dopamine level contributes to hydroxyl radical generation and subsequent neurodegeneration in the striatum in 3-nitropropionic acid-induced Huntington's disease in ratsQ45293625
Energy metabolism defects in Huntington's disease and effects of coenzyme Q10.Q45293805
Evidence for a defect in NADH: ubiquinone oxidoreductase (complex I) in Huntington's diseaseQ45294442
Coenzyme Q10 serum levels in Huntington's diseaseQ45294694
Complex I defect in muscle from patients with Huntington's disease.Q45295738
Huntingtin protein colocalizes with lesions of neurodegenerative diseases: An investigation in Huntington's, Alzheimer's, and Pick's diseasesQ45295797
Behavioural abnormalities and selective neuronal loss in HD transgenic mice expressing mutated full-length HD cDNA.Q45296558
Dopamine modulates the susceptibility of striatal neurons to 3-nitropropionic acid in the rat model of Huntington's disease.Q45296860
Biochemical abnormalities and excitotoxicity in Huntington's disease brain.Q45297145
Neuropathological classification of Huntington's diseaseQ45297167
Intranuclear inclusions and neuritic aggregates in transgenic mice expressing a mutant N-terminal fragment of huntingtinQ45297223
Mitochondrial respiration and ATP production are significantly impaired in striatal cells expressing mutant huntingtinQ45297269
A YAC mouse model for Huntington's disease with full-length mutant huntingtin, cytoplasmic toxicity, and selective striatal neurodegeneration.Q45298582
Effect of coenzyme Q10 and vitamin E on brain energy metabolism in the animal model of Huntington's diseaseQ45299091
Oxidative damage to mitochondrial DNA in Huntington's disease parietal cortexQ45299186
Striatal modulation of cAMP-response-element-binding protein (CREB) after excitotoxic lesions: implications with neuronal vulnerability in Huntington's disease.Q45299845
Creatine in Huntington disease is safe, tolerable, bioavailable in brain and reduces serum 8OH2'dG.Q45299923
Mutant huntingtin aggregates impair mitochondrial movement and trafficking in cortical neuronsQ45300079
Differential contributions of Caenorhabditis elegans histone deacetylases to huntingtin polyglutamine toxicity.Q45300254
Oxidative stress in skin fibroblasts cultures of patients with Huntington's disease.Q45302342
Transcriptional repression of PGC-1alpha by mutant huntingtin leads to mitochondrial dysfunction and neurodegeneration.Q45302702
Thermoregulatory and metabolic defects in Huntington's disease transgenic mice implicate PGC-1alpha in Huntington's disease neurodegenerationQ45302924
Mitochondrial-dependent Ca2+ handling in Huntington's disease striatal cells: effect of histone deacetylase inhibitors.Q45302980
P433issue3
P921main subjectmitochondrionQ39572
Huntington's diseaseQ190564
pathophysiologyQ1135939
P304page(s)217-226
P577publication date2010-06-01
P1433published inJournal of Bioenergetics and BiomembranesQ5044470
P1476titleMitochondrial functional alterations in relation to pathophysiology of Huntington's disease
P478volume42

Reverse relations

cites work (P2860)
Q26853356Cell signaling and mitochondrial dynamics: Implications for neuronal function and neurodegenerative disease
Q45289712Degeneration of the cerebellum in Huntington's disease (HD): possible relevance for the clinical picture and potential gateway to pathological mechanisms of the disease process.
Q34137813Distribution of GABAergic interneurons and dopaminergic cells in the functional territories of the human striatum
Q57178431Huntington's disease: the coming of age
Q51055442Impaired mitochondrial oxidative phosphorylation in the peroxisomal disease X-linked adrenoleukodystrophy.
Q36124905Mitochondria: the next (neurode)generation
Q37749622Mitochondrial matters in Huntington disease
Q27002338Physiological consequences of complex II inhibition for aging, disease, and the mKATP channel
Q38855572Quercetin improves the activity of the ubiquitin-proteasomal system in 150Q mutated huntingtin-expressing cells but exerts detrimental effects on neuronal survivability
Q37408287The Association of VDAC with Cell Viability of PC12 Model of Huntington's Disease.
Q22306292The importance of integrating basic and clinical research toward the development of new therapies for Huntington disease

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